In recent years, the understanding about the different clinical phenotypes, diagnostic and prognostic factors of myelin oligodendrocyte glycoprotein-antibody-associated disorders (MOGAD) has significantly increased. However, there is still lack of evidence-based treatment protocols for acute attacks and children with a relapsing course of the disease. Currently used acute and maintenance treatment regimens are derived from other demyelinating central nervous system diseases and are mostly centre-specific. Therefore, this part of the Paediatric European Collaborative Consensus attempts to provide recommendations for acute and maintenance therapy based on clinical experience and evidence available from mainly retrospective studies. In the acute attack, intravenous methylprednisolone (IVMP) leads to a favourable outcome in the majority of patients and can be followed by tapering of oral steroids up to a maximum of three months to maintain the benefit of acute treatment by suppressing disease activity. Intravenous immunoglobulins (IVIG) and plasmapheresis constitute second-line therapies in case of insufficient response to IVMP. After a first relapse, maintenance treatment should be started in order to prevent further relapses and the possibility of permanent sequelae. Four first-line therapies consisting of rituximab (RTX), azathioprine, mycophenolate mofetil or monthly IVIG have been identified by the consensus group. In case of further relapses despite maintenance treatment, the consensus group recommends treatment escalation with RTX or IVIG, followed by combining those two, and ultimately adding maintenance oral steroids. Many open questions remain which need to be addressed in further international prospective evaluation of MOGAD treatment. This international collaboration is essential to expand the state of current knowledge.

中文翻译：

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欧盟儿科 MOG 联盟共识：第 5 部分 – 儿科髓鞘少突胶质细胞糖蛋白抗体相关疾病的治疗

近年来，对髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）的不同临床表型、诊断和预后因素的认识显着增加。然而，对于急性发作和疾病复发的儿童，仍然缺乏循证治疗方案。目前使用的急性和维持治疗方案源自其他脱髓鞘中枢神经系统疾病，并且大多是针对中心的。因此，欧洲儿科协作共识的这一部分试图根据临床经验和主要回顾性研究提供的证据为急性和维持治疗提供建议。在急性发作时，静脉注射甲基强的松龙 (IVMP) 可使大多数患者获得良好的结果，随后可逐渐减少口服类固醇最多三个月，以通过抑制疾病活动来维持急性治疗的益处。在对 IVMP 反应不足的情况下，静脉注射免疫球蛋白 (IVIG) 和血浆置换构成二线治疗。第一次复发后，应开始维持治疗，以防止进一步复发和永久性后遗症的可能性。共识小组已经确定了四种一线疗法，包括利妥昔单抗 (RTX)、硫唑嘌呤、霉酚酸酯或每月一次的 IVIG。如果维持治疗后进一步复发，共识小组建议使用 RTX 或 IVIG 升级治疗，然后将这两者结合起来，并最终添加维持性口服类固醇。许多悬而未决的问题需要在对 MOGAD 治疗的进一步国际前瞻性评估中加以解决。这种国际合作对于扩大现有知识的状态至关重要。